CPAP and Bilevel PAP

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Non-Invasive Ventilation in Children

CPAP (continuous positive airway pressure) and Bilevel PAP (bi-level positive airway pressure) systems deliver air via contoured
nasal or full-face masks to maintain a set airway pressure. This results in increases in the functional residual capacity
(the volume of air in the lungs at the end of expiration) and/or tidal volume (volume of air in each breath). These technologies
are used in both acute and chronic care settings, including the home environment. Their most common use in children is for
obstructive sleep apnea.

CPAP delivers a constant flow of pressurized air throughout the breathing cycle, resulting in increased mean airway pressure
and functional residual capacity, but does not impact tidal volume. Bilevel PAP provides two different levels of pressure:
greater pressure during inspiration (IPAP) to improve tidal volume and a lower pressure during exhalation (EPAP) to increase
mean airway pressure and functional residual capacity. Bilevel PAP can also be set to provide “back-up breaths” to maintain
a certain minimum number of breaths per minute.

Introducing and maintaining use of these technologies can be a challenge for children and their caregivers, in ensuring tolerance
and effectiveness and minimizing side effects. Patients and their families should expect the need for high bedside caregiver
presence, at least initially. One to three weeks of gradual ‘desensitization’ to get used to breathing with the machine is
often required. Rarely, sedation is needed to help children tolerate the equipment.

Indications

CPAP or Bilevel PAP may be offered to children whose respiratory systems are compromised by weakened respiratory muscles,
weakened respiratory drive, airway obstruction, injury to lung tissue, or underdeveloped lungs. For patients with chronic
respiratory problems, they are usually used for 6 to 10 hours at night. In acute settings, they may be used as an alternative
to endotracheal intubation or in the weaning process from mechanical ventilation.

Impending respiratory muscle fatigue usually as part of acute respiratory illnesses

Asthma

Bronchiolitis

Pneumonia

Possible use for children with central hypoventilation syndrome (CHS); children with CHS demonstrate abnormal breathing patterns with symptoms of cyanosis, apnea (absence of airflow resulting
in respiratory pause), and hypopnea (shallow breathing episode)

Alternatives

For some patients, alternatives to Bilevel PAP and CPAP include:

Removal of the tonsils and adenoids (adenotonsillectomy) for OSA

Weight loss for patients with obesity

Oral appliances, although data in children is limited and use of the devices can impact craniofacial growth

Airway surgery such as septoplasty (alteration of the nasal septum), nasal turbinate reduction, uvulopalatopharyngoplasty
(alteration of the back of the palate and upper airway), and lingual tonsillectomy

Jaw surgery (maxillary or mandibular advancement surgery)

Equipment

CPAP-Bilevel PAP machines deliver a stream of heated, humidified, compressed air via a hose to a nasal pillow, nasal mask,
full-face mask, or hybrid. This “splints” the airway (keeps it open with air pressure) so that unobstructed breathing becomes
possible, reducing and/or preventing apneas (breathing stoppages) and hypopneas (shallow breathing). The masks are secured
to the patient using a headband or similar attachment device.

Flow generator (PAP machine) provides the airflow. The machines are fairly small and quiet, weighing from 6 to 12 lbs. including cables
and cords. They are portable, although most pediatric patients use them solely at home during sleep.

Hose connects the flow generator to the mask interface with the airway.

Mask (nasal, full face mask or nasal pillows) provides the connection to the user's airway. For non-invasive ventilation to be
effective, a good mask fit and seal are crucial

Optional Equipment

Humidifier adds moisture and heat to inhaled air. Heated humidified air is very important for the comfortable use of CPAP/Bilevel PAP
and to avoid side effects such as nose bleeds, dry mouth, voice changes, cough, and congestion

Mask liners may be used to prevent excess air leakage and to reduce skin irritation and dermatitis

Flexible chin straps may be used to help the patient avoid breathing through the mouth (full-face masks prevent this), thereby keeping a closed
pressure system. The straps are elastic enough that the patient can easily open his/her mouth if necessary. Modern straps
use a quick-clip instant fit. Velcro-type adjustments allow for quick size adjusting, before or after the machine is turned
on.

The machines supply air at a prescribed pressure (also called the titrated pressure), usually measured in cm of H2O. Most
machines supply room air but, if the patient is experiencing suboptimal oxygen levels, oxygen can be delivered into the machine
or mask using a special adapter.

Care/Maintenance

As with any therapy, caregivers must be aware of potential complications and side effects. The masks may cause excoriation
at the points of skin contact and acneiform rashes. Application of protective barriers, such as hydrocolloid dressings, and
use of antibiotic creams may resolve these issues. Alternating between different types of masks may also limit skin problems.
Eye irritation related to the mask or leaking air is comforted with lubricating drops, correcting the leak and/or increasing
humidification.

Poor mask fit contributes to skin and eye irritation usually from leaks around the mask. Minor leaks are acceptable as long
as they do not alter the machine’s effectiveness. If too tight, the mask may impact growth of the facial bones. A better approach
for persistent leaks is to try various size masks to find the best fit.

The care and maintenance required for PAP machines varies with the type and conditions of use and are typically spelled out
in an instruction manual. Manufacturers recommend that the end-user perform daily and weekly maintenance. Units must be checked
regularly for wear and tear and kept clean. Most masks have a lifespan of 3-6 months, airfilters in the machine last for 1-3
months and hoses usually last for 6-12 months. Inappropriate care of the equipment will result in loss of effectiveness.

Challenges/Side Effects

Common complications/side effects include:

Claustrophobia

Eye irritation

Skin irritation and sores over the bridge of the nose

Nasal congestion and sore or dry mouth

Noise that interferes with sleep (usually a sign of excessive air leak)

Nosebleeds

Upper respiratory infections

Aspiration in children with impaired swallow function

Strategies for Successful Use of Bilevel PAP in Children

Consultation with a respiratory therapist or polysomnography technician to find the correct mask and strategies for adjusting
to the equipment is recommended.

Select a comfortable mask or proper size. It should be neither too tight nor too loose, and it should not leak air when attached
to the hose and compressor.

Monitor and make appropriate adjustments in fit and CPAP/Bilevel PAP settings.

Use of protective barriers for skin may be considered if there is skin irritation.

Patients using nightly CPAP or Bilevel PAP should be seen by their health care provider regularly. Initial follow-ups may
be as frequent as 1-2 times per month. Once the patient has adjusted to therapy, annual follow-up may suffice. [Epstein: 2009] Rationale for this is based on the fact that children grow and this results in change in airway size, pressure needs as well
as severity of illness. Monitoring and addressing therapeutic side effects is also a part of the follow up evaluation. Patients
with progressive neuromuscular diseases may have gradual worsening of their weakness resulting in worsening of respiratory
function, swallow dysfunction and kyphoscoliosis. At some point of illness progression, patients with neuromuscular disorders
may no longer be suitable candidates for continued use of non-invasive ventilation.

Total costs to purchase CPAP-Bilevel PAP systems are estimated at $1,000 to $5,000. Patients can also rent the equipment.
Many major insurers, including Medicaid and Medicare, cover the cost of the technology for a range of diagnoses. Clinicians
and patients are urged to contact the patient’s insurer to determine their specific guidelines.
Although there is sufficient medical literature to support therapeutic effectiveness, FDA approval of pediatric use of home
CPAP-Bilevel PAP equipment is limited. Home care companies, in compliance with such guidelines, may refuse to cover certain
patients, resulting in difficulty with patient access to care.

Resources

Information & Support

For Parents and Patients

American Sleep Apnea AssociationA non-profit organization that promotes awareness of sleep apnea, works for continuing improvements in treatments for this
serious disorder, and advocates for the interests of sleep apnea patients.

Nasal CPAP (MedlinePlus)Brief general article about using nasal CPAP, from the National Library of Medicine's MedlinePlus